Although choledochal cysts are present at birth, they are
increasingly diagnosed in adults and now comprise 20% of all new
cases. Choledochal cysts are inflammatory in nature. Left
untreated, this frequently lead to recurrent cholangitis
(inflammation and infection in the bile ducts) or pancreatitis
(inflammation of the pancreas).

Bile duct surgery with total cyst removal is the definitive
treatment for choledochal cysts. In earlier decades, an operation
known as cystenterostomy was performed that only drained the cyst
and the bilary reconstruction left the cyst behind. That
surgery proved ineffective, leaving many patients with recurrent
cholangitis and chronic inflammation in the remaining cyst, and the
substantial risk of malignant transformation into
cholangiocarcinoma (bile duct cancer).

Today, the surgical treatment choledochal cysts cyst is
completed excised followed by reconstruction of the biliary
tree with hepaticojejunostomy in a Roux-en-Y fashion has been
widely accepted as the procedure of choice in treating and has
been

Future complications include cholangitis and a 2% risk of
malignancy, which may develop in any part of the biliary tree.